- Email: [email protected]
Cervical ependymoma presenting with brainstem and cerebellar signs: case report
Cervical ependymoma presenting with brainstem and cerebellar signs
ACKNOWLEDGEMENTS We are grateful to Dr. S. Kusunoki, Department of Neurology, Tokyo University School of Medicine for the examination of serum autoantibodies against ganglioside, to Dr. M. Shibuya, Department of Physiology (II), Showa University School of Medicine, for advice on scientific writing, and to Mary Beth Shibuya for advice on medical English.
389
indicates the need to include cervical imaging well below the foramen magnum in these circumstances. ª 2003 Elsevier Science Ltd. All rights reserved. Journal of Clinical Neuroscience (2003) 10(3), 389–391 ª 2003 Elsevier Science Ltd. All rights reserved. doi:10.1016/S0967-5868(03)00008-0
Keywords: spinal cord ependymoma, oedema Received 31 August 2001 Accepted 19 December 2001
REFERENCES 1. Peppercorn MA, Goldman P. The role of intestinal bacteria in the metabolism of salicylazosulfapyridine. J Pharmacol Exp Ther 1972; 181: 555–562. 2. Wallace JW. Neurotoxicity associated with a reduction to sulphasalazine. Practitioner 1979; 2: 276–278. 3. Price TR. Sensorimotor neuropathy with sulphasalazine. Postgrad Med J 1985; 61: 147–148. 4. Blin O, Sangla I, Jouglard J, Cottin C, Pellissier JF, Serratrice G. Axonal neuropathy and salazosulphapyridine: slow-acetylator phenotype. Rev Neurol (Paris) 1992; 148: 154–156. 5. Habal FM, Greenberg GR. Treatment of ulcerative colitis with oral 5-aminosalicylic acid including patients with adverse reactions to sulfasalazine. Am J Gastroenterol 1988; 83: 15–19. 6. Woodward DK. Peripheral neuropathy and mesalazine. BMJ 1989; 299: 1224. 7. Lossos A, River Y, Eliakim A, Steiner I. Neurologic aspects of inflammatory bowel disease. Neurology 1995; 45: 416–421. 8. Konagaya Y, Konagaya M, Takayanagi T. Chronic polyneuropathy and ulcerative colitis. Jpn J Med 1989; 28: 72–74. 9. Okiyama R, Yamada M, Tamaki M, Orimo S, Arai M. Mononeuropathy multiplex with ulcerative colitis. Intern Med 1993; 32: 651–654. 10. Chad DA, Smith TW, DeGirolami U, Hammer K. Perineuritis and ulcerative colitis. Neurology 1986; 36: 1377–1379. 11. Klotz U. Clinical pharmacokinetics of sulphasalazine, it’s metabolites and other prodrugs of 5-aminosalicylic acid. Clin Pharmacokinet 1985; 10: 285–302. 12. Das KM, Eastwood MA, McManus JPA, Sircus W. Adverse reactions during salicylazosulfapyridine therapy and the relation with drug metabolism and acetylator phenotype. N Engl J Med 1973; 289: 491–495. 13. Azad Khan AK, Nurazzaman M, Truelove SC. The effect of the acetylator phenotype on the metabolism of sulphasalazine in man. J Med Genet 1983; 20: 30–36. 14. Hanauer SB, Stathopoulos G. Risk-benefit assessment of drugs used in the treatment of inflammatory bowel disease. Drug Safety 1991; 6: 192–219. 15. Le Quesne PM. Neuropathy due to drugs. In: Dyck PJ, Thomas PK (eds) Peripheral Neuropathy, First edn. Saunders, Philadelphia 1993; 1571–1581.
Cervical ependymoma presenting with brainstem and cerebellar signs: case report
Correspondence to: Roy G. Beran, Department of Neurology, Liverpool Hospital, Elizabeth Drive, Liverpool NSW 2170, Australia. Tel.: +02-98283646; Fax: +02-98283648; E-mail: [email protected]
INTRODUCTION This case report highlights the need to consider cervical pathology as a possible cause of intracranial signs and symptoms, despite there being no previous report of an ependymoma causing intracranial features purely consequent to extensive oedema. The ependyma is a single layer of epithelium lining the ventricular spaces of the brain and extending down the centre of the spinal cord. Ependymomas, which are generally slow-growing and histologically benign, are derived from this single layer of cells. Early in life (first 2 decades), they typically occur in the fourth ventricle, where they constitute between 5 and 10% of primary brain tumours in this age group. In later years, the most common location is the spinal cord, where they constitute a large proportion of primary intraspinal neoplasms.1;2 Microscopically, ependymomas are composed of cells with rather regular, round to oval or “carrot-shaped” nuclei with abundant granular chromatin. Between the nuclei there is a fine fibrillary background that may be very dense. Helpful diagnostic features include ependymal canals and rosettes, in which tumour cells create arrays that resemble ependymal canals, and perivascular ependymal pseudorosettes, in which there is a very dense array of long, delicate ependymal processes inserted into the wall of a blood vessel, producing a prominent nucleus-free halo around the vessel. Blepharoplasts, which are the basal bodies of cilia, are stained by PTAH and are pathognomonic if present. Immunocytochemically, about 50% of ependymomas can be shown to contain the neuroglial intermediate filament glial fibrillary acidic protein (GFAP). Most tumours are well-differentiated, but anaplastic tumours occur, the most anaplastic of which resemble glioblastomas.3 CASE REPORT
1 Domit A. Azar1 MBBS(HONS) MBBS(HONS), Roy G. Beran 2 Toos Sachinwalla FRANZCR
MD FRACP FRCP,
1 Department of Neurology, Liverpool Hospital, Sydney, Australia, 2Department of Radiology, Liverpool Hospital, Sydney, Australia
Summary This case report demonstrates cervical spinal cord pathology which presented with brainstem and cerebellar signs consequent to the peritumoural oedema that extended rostrally to the pontomedullary junction. A Medline search of the literature back to 1960 failed to produce any previous report of a cervical ependymoma presenting with brainstem and cerebellar signs purely consequent to oedema. This case highlights the need to look further afield when presented with the scenario of clinical features of a brainstem lesion with only oedema apparent on cranial imaging. It
ª 2003 Elsevier Science Ltd. All rights reserved.
A 50-year-old caucasian man presented with a 6 week history of gait disturbance, non-specific sensory symptoms throughout his body and pins and needles paraesthesia in both hands and feet. He described a “tingling” feeling in his legs, difficulty with manual dexterity such that he dropped objects and complained of incoordination. There was a past history of head injury from which he made a full recovery with no neurological deficit. Neurological examination revealed nystagmus of the abducted right eye with limited adduction of the left eye, suggesting an internuclear ophthalmoplegia due to a brainstem lesion. He had ataxia of gait, falling to the right, without other features of cerebellar disease such as dysmetria, dysarthria, dysdiadochokinesis or intention tremor. He reported non-specific sensory changes in the right lower limb which were neither dermatomal nor nerve-specific to provide extra localising features. The provisional diagnosis was of Journal of Clinical Neuroscience (2003) 10(3)
390 Azar et al.
Fig. 1 Sagittal T2 TSE section demonstrating extensive cord oedema up to the pontomedullary junction (arrow) associated with a mass from C4-5 to C7.
Fig. 3 Histopathological section of excised tumour verifying the diagnosis as ependymoma. H and E, original magnification 10.
brainstem or cerebellar lesion. There was some gliosis bilaterally in the frontal lobes from his previous head trauma which was thought to be irrelevant to his current presentation. Because of the brainstem oedema devoid of identifiable aetiology, spinal MRI was sought and revealed oedema extending rostrally into the pontomedullary junction, and caudally to the 11th thoracic vertebral level (Fig. 1). This was seen to be caused by a large intradural-intramedullary lesion, most likely an ependymoma or glioma. It extended from the midportion of the fourth cervical vertebral level to the inferior endplate level of the seventh cervical vertebra, with a cystic appearance (Fig. 2). The patient had the tumour removed following a C3 to C7 laminectomy, with resolution of the oedema and subsequent recovery of the brainstem/cerebellar signs. Histopathology revealed a benign ependymoma with clear margins suggestive of complete excision (Fig. 3), and no radiotherapy was advocated for the patient. DISCUSSION
Fig. 2 Sagittal T1-weighted post-contrast section demonstrating a mixed solid/cystic intradural-intramedullary mass extending from C4-5 to C7 (between arrows).
brainstem/cerebellar deficit and cranial MRI with particular reference to the posterior fossa was requested. This revealed significant oedema within the brainstem without evidence of a Journal of Clinical Neuroscience (2003) 10(3)
This case demonstrates cervical spinal cord pathology which presented with brainstem and cerebellar signs consequent to the peritumoural oedema that extended rostrally to the pontomedullary junction. The patient’s signs were most likely due to the direct effects of oedema on the proximal left medial longitudinal fasciculus, as well as the inferior pathway into the right cerebellar hemisphere (via the inferior cerebellar peduncle). The diffuse nature of the oedema also accounts for the vague sensory symptoms and signs that were part of this presentation. A Medline search of the literature back to 1960 failed to produce any previous report of a cervical ependymoma presenting with brainstem and cerebellar signs consequent to oedema. There were references to ependymomas and peritumoural oedema with intracranial ependymomas4–8 but not associated with false-localising signs. ª 2003 Elsevier Science Ltd. All rights reserved.
Multiple schwannomas of the sciatic nerve
There were numerous references to spinal cord ependymomas,9;10 but none causing what amounts to distant intracranial false-localising signs consequent to oedema. This case highlights the need to look further afield when presented with the scenario of clinical features of a brainstem lesion with only oedema apparent on cranial imaging. It indicates the need to include cervical imaging well below the foramen magnum in these circumstances. REFERENCES 1. Slooff JL et al. Primary Intramedullary Tumors of the Spinal Cord and Filum Terminale. W.B. Saunders Company, Philadelphia 1964. 2. Ilgren EB et al. Ependymomas: A clinical and pathological study. Part 1 Biologic features. Clin Neuropathol 1984; 3: 113. 3. Robbins S. The nervous system. In: Cotran R, Kumar V, Robbins S (eds) Pathologic Basis of Disease. fourth ed. W.B. Saunders Company, Philadelphia 1989: 1417. 4. Comi AM, Backstrom JW, Burger PC, Duffner PK. Clinical and neuroradiologic findings in infants with intracranial ependymomas. Pediatric Oncology Group. Pediatr Neurol 1998; 18(1): 23–29. 5. Grabb PA, Lunford LD, Albright AL, Kondziolka D, Flickinger JC. Stereotactic radiosurgery fir glial neoplasms of childhood. Neurosurgery 1996; 38(4): 696– 701, discussion 702. 6. Vernet O, Farmer JP, Meagher-Villemure K, Montes JL. Supratentorial ectopic ependymoma. Can J Neurol Sci 1995; 22(4): 316–319. 7. Armington WG, Osborn AG, Cubberly DA et al. Supratentorial ependymoma: CT appearance. Radiology 1985; 157(2): 367–372. 8. Hanakita J, Handa H. Clinical and CT findings of supratentorial ependymomas and ependymoblastomas. No Shinkei Geka 1984; 12(3 Suppl): 253–260. 9. Newton B, Rea GL. Lhermitte’s sign as a presenting symptom of primary spinal cord tumor. J Neurooncol 1996; 29(2): 183–188. 10. Katoh S, Ikata T, Inoue A, Takahashi M. Intradural extramedullary ependymoma. A case report. Spine 1995; 20(18): 2036–2038.
Multiple schwannomas of the sciatic nerveq J. Huang BSC(MED), R. Mobbs C. Teo MB BS MD FRACS
BSC(MED) MB BS,
391
INTRODUCTION Single schwannoma, a rare benign tumour of nerve sheath cells of neural crest origin, is the most common of all peripheral tumours.1 Multiple schwannomas of the peripheral and central nervous system are, however, uncommon. Multiple schwannomas may arise from the cranial nerves, spinal roots, the brachial and lumbar-sacral plexus or major peripheral nerves. These tumours are often solitary and encapsulated. The treatment for solitary schwannoma is to enucleate from the nerve of origin and this can be performed with minimal loss of function in that nerve.1 We report a very rare case of schwannomatosis found along the right sciatic nerve with no associated neurofibromatosis. CASE REPORT An 18-year-old female asthmatic ballet dancer presented with right leg pain. There was no preceding history of trauma. The pain started in the right buttock area with radiation down the back of her leg to the base of her foot, progressive over an 18-month period. She had weakness of her thigh extensors and ankle plantar flexion. Multiple opinions were sought. CT and MR scans of her lumbo-sacral spine were normal. Head CT and MR scans were normal. The patient had never noticed any subcutaneous lumps of nodules. There was no history of tinnitus, hearing loss or vestibular problems. There were no cafe-au-lait spots and ophthalmic examination revealed no visible evidence of cataracts. There was no family history of schwannomatosis or NF2 (neurofibromatosis Type 2, formerly “central neurofibromatosis”). Two weeks prior to presentation there was worsening of her leg pain and she developed weakness of ankle dorsiflexion at 4-/5. Nerve conduction studies showed a mono-neuropathy of the sciatic nerve with slowed conduction along the entire length of the sciatic nerve. MR scans of the gluteal and thigh region demonstrated a generalised increase in diameter of the sciatic nerve with multiple lesions on her sciatic nerve (Figs. 1 and 2). There was loss of right gluteal muscle mass. Her superior and inferior gluteal nerves were not affected with tumour, but there was presumably compression neuropathy of these nerves from the grossly enlarged sciatic nerve.
Center for Minimally Invasive Surgery, The Prince of Wales Private Hospital, Division of Neurosurgery, NSW, Australia
Summary Schwannomas are rare benign tumours of nerve sheath cells of neural crest origin. Often these tumours are solitary and encapsulated. Multiple schwannomas can arise from the peripheral nervous system including cranial nerves, spinal roots, the brachial and lumbar–sacral plexus or major peripheral nerves. We report an extremely rare case of schwannomatosis of the sciatic nerve in a young female and include a comprehensive literature review. Treatment options are discussed. ª 2003 Elsevier Science Ltd. All rights reserved. Journal of Clinical Neuroscience (2003) 10(3), 391–393 ª 2003 Elsevier Science Ltd. All rights reserved. doi:10.1016/S0967-5868(03)00021-3
Keywords: multiple schwannomas, schwannomatosis, sciatic nerve Received 26 October 2002 Accepted 26 November 2002 Correspondence to: C. Teo, Suite 3, Level 7, POWP Hospital, Barker Street, Randwick, 2031, Australia. q There were no sources of support or grants of any kind used in the production of this case report and review.
ª 2003 Elsevier Science Ltd. All rights reserved.
Fig. 1
T2WI coronal MRI of the right sciatic nerve: note the multiple lesions.
Journal of Clinical Neuroscience (2003) 10(3)
ACKNOWLEDGEMENTS We are grateful to Dr. S. Kusunoki, Department of Neurology, Tokyo University School of Medicine for the examination of serum autoantibodies against ganglioside, to Dr. M. Shibuya, Department of Physiology (II), Showa University School of Medicine, for advice on scientific writing, and to Mary Beth Shibuya for advice on medical English.
389
indicates the need to include cervical imaging well below the foramen magnum in these circumstances. ª 2003 Elsevier Science Ltd. All rights reserved. Journal of Clinical Neuroscience (2003) 10(3), 389–391 ª 2003 Elsevier Science Ltd. All rights reserved. doi:10.1016/S0967-5868(03)00008-0
Keywords: spinal cord ependymoma, oedema Received 31 August 2001 Accepted 19 December 2001
REFERENCES 1. Peppercorn MA, Goldman P. The role of intestinal bacteria in the metabolism of salicylazosulfapyridine. J Pharmacol Exp Ther 1972; 181: 555–562. 2. Wallace JW. Neurotoxicity associated with a reduction to sulphasalazine. Practitioner 1979; 2: 276–278. 3. Price TR. Sensorimotor neuropathy with sulphasalazine. Postgrad Med J 1985; 61: 147–148. 4. Blin O, Sangla I, Jouglard J, Cottin C, Pellissier JF, Serratrice G. Axonal neuropathy and salazosulphapyridine: slow-acetylator phenotype. Rev Neurol (Paris) 1992; 148: 154–156. 5. Habal FM, Greenberg GR. Treatment of ulcerative colitis with oral 5-aminosalicylic acid including patients with adverse reactions to sulfasalazine. Am J Gastroenterol 1988; 83: 15–19. 6. Woodward DK. Peripheral neuropathy and mesalazine. BMJ 1989; 299: 1224. 7. Lossos A, River Y, Eliakim A, Steiner I. Neurologic aspects of inflammatory bowel disease. Neurology 1995; 45: 416–421. 8. Konagaya Y, Konagaya M, Takayanagi T. Chronic polyneuropathy and ulcerative colitis. Jpn J Med 1989; 28: 72–74. 9. Okiyama R, Yamada M, Tamaki M, Orimo S, Arai M. Mononeuropathy multiplex with ulcerative colitis. Intern Med 1993; 32: 651–654. 10. Chad DA, Smith TW, DeGirolami U, Hammer K. Perineuritis and ulcerative colitis. Neurology 1986; 36: 1377–1379. 11. Klotz U. Clinical pharmacokinetics of sulphasalazine, it’s metabolites and other prodrugs of 5-aminosalicylic acid. Clin Pharmacokinet 1985; 10: 285–302. 12. Das KM, Eastwood MA, McManus JPA, Sircus W. Adverse reactions during salicylazosulfapyridine therapy and the relation with drug metabolism and acetylator phenotype. N Engl J Med 1973; 289: 491–495. 13. Azad Khan AK, Nurazzaman M, Truelove SC. The effect of the acetylator phenotype on the metabolism of sulphasalazine in man. J Med Genet 1983; 20: 30–36. 14. Hanauer SB, Stathopoulos G. Risk-benefit assessment of drugs used in the treatment of inflammatory bowel disease. Drug Safety 1991; 6: 192–219. 15. Le Quesne PM. Neuropathy due to drugs. In: Dyck PJ, Thomas PK (eds) Peripheral Neuropathy, First edn. Saunders, Philadelphia 1993; 1571–1581.
Cervical ependymoma presenting with brainstem and cerebellar signs: case report
Correspondence to: Roy G. Beran, Department of Neurology, Liverpool Hospital, Elizabeth Drive, Liverpool NSW 2170, Australia. Tel.: +02-98283646; Fax: +02-98283648; E-mail: [email protected]
INTRODUCTION This case report highlights the need to consider cervical pathology as a possible cause of intracranial signs and symptoms, despite there being no previous report of an ependymoma causing intracranial features purely consequent to extensive oedema. The ependyma is a single layer of epithelium lining the ventricular spaces of the brain and extending down the centre of the spinal cord. Ependymomas, which are generally slow-growing and histologically benign, are derived from this single layer of cells. Early in life (first 2 decades), they typically occur in the fourth ventricle, where they constitute between 5 and 10% of primary brain tumours in this age group. In later years, the most common location is the spinal cord, where they constitute a large proportion of primary intraspinal neoplasms.1;2 Microscopically, ependymomas are composed of cells with rather regular, round to oval or “carrot-shaped” nuclei with abundant granular chromatin. Between the nuclei there is a fine fibrillary background that may be very dense. Helpful diagnostic features include ependymal canals and rosettes, in which tumour cells create arrays that resemble ependymal canals, and perivascular ependymal pseudorosettes, in which there is a very dense array of long, delicate ependymal processes inserted into the wall of a blood vessel, producing a prominent nucleus-free halo around the vessel. Blepharoplasts, which are the basal bodies of cilia, are stained by PTAH and are pathognomonic if present. Immunocytochemically, about 50% of ependymomas can be shown to contain the neuroglial intermediate filament glial fibrillary acidic protein (GFAP). Most tumours are well-differentiated, but anaplastic tumours occur, the most anaplastic of which resemble glioblastomas.3 CASE REPORT
1 Domit A. Azar1 MBBS(HONS) MBBS(HONS), Roy G. Beran 2 Toos Sachinwalla FRANZCR
MD FRACP FRCP,
1 Department of Neurology, Liverpool Hospital, Sydney, Australia, 2Department of Radiology, Liverpool Hospital, Sydney, Australia
Summary This case report demonstrates cervical spinal cord pathology which presented with brainstem and cerebellar signs consequent to the peritumoural oedema that extended rostrally to the pontomedullary junction. A Medline search of the literature back to 1960 failed to produce any previous report of a cervical ependymoma presenting with brainstem and cerebellar signs purely consequent to oedema. This case highlights the need to look further afield when presented with the scenario of clinical features of a brainstem lesion with only oedema apparent on cranial imaging. It
ª 2003 Elsevier Science Ltd. All rights reserved.
A 50-year-old caucasian man presented with a 6 week history of gait disturbance, non-specific sensory symptoms throughout his body and pins and needles paraesthesia in both hands and feet. He described a “tingling” feeling in his legs, difficulty with manual dexterity such that he dropped objects and complained of incoordination. There was a past history of head injury from which he made a full recovery with no neurological deficit. Neurological examination revealed nystagmus of the abducted right eye with limited adduction of the left eye, suggesting an internuclear ophthalmoplegia due to a brainstem lesion. He had ataxia of gait, falling to the right, without other features of cerebellar disease such as dysmetria, dysarthria, dysdiadochokinesis or intention tremor. He reported non-specific sensory changes in the right lower limb which were neither dermatomal nor nerve-specific to provide extra localising features. The provisional diagnosis was of Journal of Clinical Neuroscience (2003) 10(3)
390 Azar et al.
Fig. 1 Sagittal T2 TSE section demonstrating extensive cord oedema up to the pontomedullary junction (arrow) associated with a mass from C4-5 to C7.
Fig. 3 Histopathological section of excised tumour verifying the diagnosis as ependymoma. H and E, original magnification 10.
brainstem or cerebellar lesion. There was some gliosis bilaterally in the frontal lobes from his previous head trauma which was thought to be irrelevant to his current presentation. Because of the brainstem oedema devoid of identifiable aetiology, spinal MRI was sought and revealed oedema extending rostrally into the pontomedullary junction, and caudally to the 11th thoracic vertebral level (Fig. 1). This was seen to be caused by a large intradural-intramedullary lesion, most likely an ependymoma or glioma. It extended from the midportion of the fourth cervical vertebral level to the inferior endplate level of the seventh cervical vertebra, with a cystic appearance (Fig. 2). The patient had the tumour removed following a C3 to C7 laminectomy, with resolution of the oedema and subsequent recovery of the brainstem/cerebellar signs. Histopathology revealed a benign ependymoma with clear margins suggestive of complete excision (Fig. 3), and no radiotherapy was advocated for the patient. DISCUSSION
Fig. 2 Sagittal T1-weighted post-contrast section demonstrating a mixed solid/cystic intradural-intramedullary mass extending from C4-5 to C7 (between arrows).
brainstem/cerebellar deficit and cranial MRI with particular reference to the posterior fossa was requested. This revealed significant oedema within the brainstem without evidence of a Journal of Clinical Neuroscience (2003) 10(3)
This case demonstrates cervical spinal cord pathology which presented with brainstem and cerebellar signs consequent to the peritumoural oedema that extended rostrally to the pontomedullary junction. The patient’s signs were most likely due to the direct effects of oedema on the proximal left medial longitudinal fasciculus, as well as the inferior pathway into the right cerebellar hemisphere (via the inferior cerebellar peduncle). The diffuse nature of the oedema also accounts for the vague sensory symptoms and signs that were part of this presentation. A Medline search of the literature back to 1960 failed to produce any previous report of a cervical ependymoma presenting with brainstem and cerebellar signs consequent to oedema. There were references to ependymomas and peritumoural oedema with intracranial ependymomas4–8 but not associated with false-localising signs. ª 2003 Elsevier Science Ltd. All rights reserved.
Multiple schwannomas of the sciatic nerve
There were numerous references to spinal cord ependymomas,9;10 but none causing what amounts to distant intracranial false-localising signs consequent to oedema. This case highlights the need to look further afield when presented with the scenario of clinical features of a brainstem lesion with only oedema apparent on cranial imaging. It indicates the need to include cervical imaging well below the foramen magnum in these circumstances. REFERENCES 1. Slooff JL et al. Primary Intramedullary Tumors of the Spinal Cord and Filum Terminale. W.B. Saunders Company, Philadelphia 1964. 2. Ilgren EB et al. Ependymomas: A clinical and pathological study. Part 1 Biologic features. Clin Neuropathol 1984; 3: 113. 3. Robbins S. The nervous system. In: Cotran R, Kumar V, Robbins S (eds) Pathologic Basis of Disease. fourth ed. W.B. Saunders Company, Philadelphia 1989: 1417. 4. Comi AM, Backstrom JW, Burger PC, Duffner PK. Clinical and neuroradiologic findings in infants with intracranial ependymomas. Pediatric Oncology Group. Pediatr Neurol 1998; 18(1): 23–29. 5. Grabb PA, Lunford LD, Albright AL, Kondziolka D, Flickinger JC. Stereotactic radiosurgery fir glial neoplasms of childhood. Neurosurgery 1996; 38(4): 696– 701, discussion 702. 6. Vernet O, Farmer JP, Meagher-Villemure K, Montes JL. Supratentorial ectopic ependymoma. Can J Neurol Sci 1995; 22(4): 316–319. 7. Armington WG, Osborn AG, Cubberly DA et al. Supratentorial ependymoma: CT appearance. Radiology 1985; 157(2): 367–372. 8. Hanakita J, Handa H. Clinical and CT findings of supratentorial ependymomas and ependymoblastomas. No Shinkei Geka 1984; 12(3 Suppl): 253–260. 9. Newton B, Rea GL. Lhermitte’s sign as a presenting symptom of primary spinal cord tumor. J Neurooncol 1996; 29(2): 183–188. 10. Katoh S, Ikata T, Inoue A, Takahashi M. Intradural extramedullary ependymoma. A case report. Spine 1995; 20(18): 2036–2038.
Multiple schwannomas of the sciatic nerveq J. Huang BSC(MED), R. Mobbs C. Teo MB BS MD FRACS
BSC(MED) MB BS,
391
INTRODUCTION Single schwannoma, a rare benign tumour of nerve sheath cells of neural crest origin, is the most common of all peripheral tumours.1 Multiple schwannomas of the peripheral and central nervous system are, however, uncommon. Multiple schwannomas may arise from the cranial nerves, spinal roots, the brachial and lumbar-sacral plexus or major peripheral nerves. These tumours are often solitary and encapsulated. The treatment for solitary schwannoma is to enucleate from the nerve of origin and this can be performed with minimal loss of function in that nerve.1 We report a very rare case of schwannomatosis found along the right sciatic nerve with no associated neurofibromatosis. CASE REPORT An 18-year-old female asthmatic ballet dancer presented with right leg pain. There was no preceding history of trauma. The pain started in the right buttock area with radiation down the back of her leg to the base of her foot, progressive over an 18-month period. She had weakness of her thigh extensors and ankle plantar flexion. Multiple opinions were sought. CT and MR scans of her lumbo-sacral spine were normal. Head CT and MR scans were normal. The patient had never noticed any subcutaneous lumps of nodules. There was no history of tinnitus, hearing loss or vestibular problems. There were no cafe-au-lait spots and ophthalmic examination revealed no visible evidence of cataracts. There was no family history of schwannomatosis or NF2 (neurofibromatosis Type 2, formerly “central neurofibromatosis”). Two weeks prior to presentation there was worsening of her leg pain and she developed weakness of ankle dorsiflexion at 4-/5. Nerve conduction studies showed a mono-neuropathy of the sciatic nerve with slowed conduction along the entire length of the sciatic nerve. MR scans of the gluteal and thigh region demonstrated a generalised increase in diameter of the sciatic nerve with multiple lesions on her sciatic nerve (Figs. 1 and 2). There was loss of right gluteal muscle mass. Her superior and inferior gluteal nerves were not affected with tumour, but there was presumably compression neuropathy of these nerves from the grossly enlarged sciatic nerve.
Center for Minimally Invasive Surgery, The Prince of Wales Private Hospital, Division of Neurosurgery, NSW, Australia
Summary Schwannomas are rare benign tumours of nerve sheath cells of neural crest origin. Often these tumours are solitary and encapsulated. Multiple schwannomas can arise from the peripheral nervous system including cranial nerves, spinal roots, the brachial and lumbar–sacral plexus or major peripheral nerves. We report an extremely rare case of schwannomatosis of the sciatic nerve in a young female and include a comprehensive literature review. Treatment options are discussed. ª 2003 Elsevier Science Ltd. All rights reserved. Journal of Clinical Neuroscience (2003) 10(3), 391–393 ª 2003 Elsevier Science Ltd. All rights reserved. doi:10.1016/S0967-5868(03)00021-3
Keywords: multiple schwannomas, schwannomatosis, sciatic nerve Received 26 October 2002 Accepted 26 November 2002 Correspondence to: C. Teo, Suite 3, Level 7, POWP Hospital, Barker Street, Randwick, 2031, Australia. q There were no sources of support or grants of any kind used in the production of this case report and review.
ª 2003 Elsevier Science Ltd. All rights reserved.
Fig. 1
T2WI coronal MRI of the right sciatic nerve: note the multiple lesions.
Journal of Clinical Neuroscience (2003) 10(3)